Lethal Hereditary Vascular Disorders: Osler-Weber-Rendu Syndrome, Ataxia-Telangiectasia, and Fabry’s Disease

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Abstract

Mucocutaneous vascular ectasia, otherwise referred to as telangiectasia, can be an important harbinger of serious systemic disease. Among a variety of acquired conditions, including hepatic cirrhosis, that are responsible for their development, are a heterogeneous group of inherited conditions that entail the development of multiple cutaneous and mucous membrane vascular lesions associated with other life-threatening complications. This chapter deals with the clinical and pathologic features of three such conditions, namely, Osler-Weber-Rendu (OWR) syndrome, ataxia-telangiectasia (AT), and Fabry’s disease (FD).

Keywords

OWR, hereditary hemorrhagic telangiectasia AT, Louis-Bar syndrome 
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Copyright information

© Springer International Publishing Switzerland 2016

Authors and Affiliations

  1. 1.Director of DermatopathologyDermatology Specialists of FloridaPanama CityUSA
  2. 2.Staff DermatopathologistAdvanced Dermatology and Cosmetic SurgeryClermontUSA
  3. 3.Professor of DermatologyMichigan State College of MedicineEast LancingUSA

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